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This chapter is from the book

This chapter is from the book

Thalassemia

Thalassemia is an autosomal-recessive group of hereditary blood disorders that is found mostly in the African-Americans, Asians, and the Middle East populations. The most common forms are

  • Heterozygous
    • Thalassemia minor or thalassemia trait (mild microcytic anemia occurs)
    • Thalassemia intermedia (splenomegaly and severe anemia are manifested)
  • Homozygous
    • Thalassemia major or Cooley's anemia (severely anemic), where patients cannot live without blood transfusions.
    • The client's red blood cells are destroyed prematurely.

The signs and symptoms associated with thalassemia are frequently associated with anemia and include

  • Pallor
  • Loss of weight
  • Hepatosplenomegaly
  • Severe anemia
  • Folic acid deficiency
  • Osteoporosis and associated fractures
  • Heart murmurs
  • Darkening of skin
  • Headache
  • Epistaxis
  • Gout
  • Bone pain
  • Hemosiderosis (excess iron in body tissues)
  • Hemochromatosis (excess iron storage resulting in cell damage)

The treatment of thalassemia revolves around maintenance of adequate hemoglobin that will allow oxygenation of tissues and prevention and treatment of complications. The following objectives will be accomplished by the nurse:

  • Monitoring ordered blood transfusion
  • Monitoring for excess hemosiderosis and hepatitis
  • Observing for signs and symptoms of infection
  • Administering ordered folic acid
  • Reinforce teaching to prevent fractures: no contact sports, slippery rugs, and so on
  • Implementing iron chelation treatment with deferoxamine (Desferal)
  • Supporting the patient and family during bone marrow transplantation
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